Pediatric neurology最新文献

{"title":"Current State of the Global Pediatric Stroke Care-Results From the Global Alliance for Pediatric Stroke Epidemiology and Resources Survey","authors":"Ilona Kopyta ,&nbsp;Manish Parakh ,&nbsp;Gabrielle deVeber ,&nbsp;Yenny C. Zuñiga Z ,&nbsp;Maria Fernanda Rios Pistoia ,&nbsp;Kevin O'Connor ,&nbsp;Fernanda Paz Balut ,&nbsp;Jenny Wilson ,&nbsp;Marilyn Tan ,&nbsp;Allyson Schenk ,&nbsp;Piotr Rodak ,&nbsp;Ankit Kumar Meena ,&nbsp;Suman Das","doi":"10.1016/j.pediatrneurol.2025.09.020","DOIUrl":"10.1016/j.pediatrneurol.2025.09.020","url":null,"abstract":"<div><h3>Background</h3><div>Pediatric stroke care varies across institutions, but global differences have not been explored. Our objective was to evaluate global variability and challenges in pediatric stroke care.</div></div><div><h3>Methods</h3><div>The Global Alliance for Pediatric Stroke Epidemiology and Resources Working Group of the International Pediatric Stroke Organization distributed a 43-question online survey to international child neurology organizations.</div></div><div><h3>Results</h3><div>A total of 135 responses were received, from Europe, North America, and Australia (n = 66), South America (n = 27) and Asia and Africa (AA; n = 42). Respondents were mainly pediatric neurologists from quaternary/tertiary teaching hospitals (&gt;90%) and worked in urban areas (85%-90%) or large cities (40%-60%). Outside of Asia, women comprised 70% of the respondents, whereas males made up the majority in Asia. Globally, pediatric neurologists manage emergency room triage and inpatient treatment stroke in most facilities (65%-68%). Experienced (&gt;20 years) pediatric stroke subspecialists were available in a minority of centers (7%-17%). Multidisciplinary teams and institutional algorithms were more common in Europe and the Americas compared to Asia (53% vs 7-11% and 80-85% vs 20%, respectively). Reduced awareness of pediatric stroke was identified as a reason for the delay in seeking care in Europe, North America, and Australia by caregivers (44%) and health providers (38%), while in AA it was 67% and 58%.</div></div><div><h3>Conclusions</h3><div>Across all regions, significant challenges in pediatric stroke management exist. Serious gaps exist in Asia related to personnel, expertise, and infrastructure. Development and improvement of local policies and resources are urgently needed in AA to improve pediatric stroke outcomes.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"174 ","pages":"Pages 101-109"},"PeriodicalIF":2.1,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145467817","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Treatment Practices for Infantile Epileptic Spasms Syndrome: Consensus and Variation in Major Pediatric Epilepsy Centers","authors":"Christina Briscoe MD, EdM ,&nbsp;Akshat Katyayan MD ,&nbsp;Chellamani Harini MD ,&nbsp;Shaun A. Hussain MD ,&nbsp;Sonam Bhalla MD ,&nbsp;Avantika Singh MD ,&nbsp;Stephanie Donatelli MD ,&nbsp;Amanda G. Sandoval Karamian MD ,&nbsp;Debopam Samanta MD ,&nbsp;Deepa Sirsi MD ,&nbsp;Eva Catenaccio MD ,&nbsp;Maria A. Planchart Ferretto MD ,&nbsp;Liu Lin Thio MD, PhD ,&nbsp;Senyene E. Hunter MD, PhD ,&nbsp;Pavuluri Spriha MD ,&nbsp;Chethan K. Rao DO, MS ,&nbsp;Sonal Bhatia MD ,&nbsp;Gozde Erdemir MD ,&nbsp;Daniel W. Shrey MD ,&nbsp;Adam L. Numis MD","doi":"10.1016/j.pediatrneurol.2025.10.005","DOIUrl":"10.1016/j.pediatrneurol.2025.10.005","url":null,"abstract":"<div><h3>Background</h3><div>Infantile epileptic spasms syndrome (IESS) is a developmental and epileptic encephalopathy that requires prompt, effective treatment to optimize outcomes. While the first therapies for IESS with adrenocorticotrophic hormone, prednisolone, or vigabatrin are widely established as a standard, we hypothesized that the treatment protocols of how these therapies should be implemented varied across medical centers.</div></div><div><h3>Methods</h3><div>The Pediatric Epilepsy Research Consortium Infantile Spasms Special Interest Group distributed a REDCap survey to 75 US epilepsy centers. Predefined treatment pathway characteristics were extracted and compared. Standard therapy regimens were defined before data collection.</div></div><div><h3>Results</h3><div>Thirty-six centers participated (48% completion rate). Most (89%, n = 32) had IESS treatment pathways, with 72% (n = 23) influenced by insurance barriers such as prior authorizations. Of these, 75% (n = 24) contributed pathways for analysis. Most protocols (88%, n = 21) recommended a standard treatment course for new-onset IESS. Of these, 63% (n = 15) endorsed a sequential approach to using hormonal therapy and vigabatrin, while 17% (n = 4) recommended combination therapy with both for all children. Thirteen centers (54%) provided recommendations for treating persistent epileptic spasms. Approaches to side-effect mitigation varied widely, with gastrointestinal prophylaxis and blood pressure control being the most common (79%, n = 19). Half of the pathways mentioned ketogenic diet (58%) or epilepsy surgery (46%).</div></div><div><h3>Conclusions</h3><div>While there was broad consensus regarding first and second therapy treatment for IESS, variability existed in using sequential versus combination therapy, third therapies, and adverse event monitoring. These findings will guide next research steps in defining key questions on sequential versus combination therapy, third line therapy, and adverse event monitoring in order to develop a standardized consensus-driven treatment protocol for IESS in the future.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"174 ","pages":"Pages 46-53"},"PeriodicalIF":2.1,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145419784","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Friedreich Ataxia","authors":"S.H. Subramony MD ,&nbsp;David R. Lynch MD, PhD","doi":"10.1016/j.pediatrneurol.2025.10.020","DOIUrl":"10.1016/j.pediatrneurol.2025.10.020","url":null,"abstract":"<div><div>With the introduction of potential new therapy for Friedreich ataxia, the disorder has taken on a new importance in the world of pediatric neurology. Originally described more than 150 years ago, large scale clinical studies have defined diagnostic criteria and the underlying mutation as a biallelic, unstable expansion of an intronic guanine adenine adenine repeat in chromosome 9. In this review, we summarize the clinical features, routine management, pathophysiology, and emerging therapies for this devastating disease. The recent approval of omaveloxolone makes recognition of Friedreich ataxia and its treatment essential for all pediatric neurologists.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"174 ","pages":"Pages 148-154"},"PeriodicalIF":2.1,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145549983","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Limited Utility for Head Ultrasounds in the Evaluation of Tuberous Sclerosis Complex","authors":"Savannah Stultz BS ,&nbsp;Darcy A. Krueger MD, PhD ,&nbsp;David N. Franz MD ,&nbsp;Paul S. Horn PhD ,&nbsp;David M. Ritter MD, PhD","doi":"10.1016/j.pediatrneurol.2025.10.011","DOIUrl":"10.1016/j.pediatrneurol.2025.10.011","url":null,"abstract":"<div><h3>Background</h3><div>Tuberous sclerosis complex (TSC) is a genetic disorder leading to multiorgan disease. Three cerebral structural manifestations in TSC are tubers, subependymal nodules, and subependymal giant cell astrocytomas (SEGAs). Head ultrasound (HUS) has been established as a diagnostic tool for early head imaging. However, the utility of HUS in detecting TSC cerebral manifestations is not known.</div></div><div><h3>Methods</h3><div>We conducted a retrospective chart review of clinical brain imaging reports in TSC patients followed by the Cincinnati Children's TSC Clinic. From the clinical reports, the presence of TSC and other lesions were recorded. 373 individuals had either a HUS (N = 42) or a magnetic resonance imaging (MRI; N = 372) before 5 years of age.</div></div><div><h3>Results</h3><div>Of the 42 patients with HUS, 24 were found to have completely normal HUS (57%). However, on the first MRI, only 3 had normal brain MRIs (13% of normal HUS). One had not had an MRI at the time of data collection and the other 20 had typical TSC lesions. No SEGA was seen on the HUS reports although 5 in the total cohort had a SEGA on MRI reports before 1 month of age. If the HUS was normal, there was a trend toward delaying the first MRI (0.2 ± 0.49 years vs 0.66 ± 0.98 years, <em>P</em> = 0.06).</div></div><div><h3>Conclusions</h3><div>Compared to the patient's initial MRI results, HUS underdetected the cerebral changes associated with TSC and a normal HUS tends to delay definitive MRI imaging. Thus, HUS has limited utility during the routine workup for TSC.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"174 ","pages":"Pages 86-90"},"PeriodicalIF":2.1,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145468459","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"First Line Management of Pediatric Status Epilepticus","authors":"Jennifer V. Gettings BMBS, FRCPC","doi":"10.1016/j.pediatrneurol.2025.10.015","DOIUrl":"10.1016/j.pediatrneurol.2025.10.015","url":null,"abstract":"","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"174 ","pages":"Page 118"},"PeriodicalIF":2.1,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145506367","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Tasks for Assessing Dystonia in Young People With Cerebral Palsy","authors":"Emma Lott BA ,&nbsp;Alyssa Rust BS ,&nbsp;Leon Dure MD ,&nbsp;Darcy Fehlings MD ,&nbsp;Toni Pearson MBBS ,&nbsp;Roser Pons MD ,&nbsp;Jonathan W. Mink MD, PhD ,&nbsp;Bhooma R. Aravamuthan MD, DPhil","doi":"10.1016/j.pediatrneurol.2025.10.010","DOIUrl":"10.1016/j.pediatrneurol.2025.10.010","url":null,"abstract":"<div><h3>Background</h3><div>Dystonia in childhood is typically associated with cerebral palsy (CP). Dystonia severity scales for CP require prolonged exam protocols with numerous tasks, often making them onerous for routine clinical use. We aimed to identify which individual tasks best approximate dystonia severity compared to the gold standard full protocol.</div></div><div><h3>Methods</h3><div>In this cross-sectional study, comprehensive exam protocol videos were taken during routine care of ambulatory people with CP age 5 years and up. Five pediatric dystonia experts reviewed individual tasks and the full protocol for dystonia using the Global Dystonia Severity Rating Scale. Experts’ written scoring justifications were qualitatively analyzed to determine commonly cited features of dystonia.</div></div><div><h3>Results</h3><div>When examining the difference in dystonia severity ratings between each task and the full protocol, seated upper extremity tasks had the lowest variance (<em>P</em> &lt; 0.05, F-test) and had lower score differences than the stand/walk/run and seated lower extremity tasks (<em>P</em> &lt; 0.05, repeated measures Friedman test). Experts most commonly identified the following movements as dystonic: wrist flexion (8.4% of all movement statements), finger flexion (7.3%), wrist ulnar deviation (6.8%), toe dorsiflexion (8.4%), ankle inversion (7.9%), and ankle plantarflexion (6.4%). Experts rated dystonic movements as more severe if they were consistently triggered by multiple stimuli (26.8% of all severity statements) or functionally impactful (20.7%).</div></div><div><h3>Conclusions</h3><div>Seated upper extremity tasks may be valuable for identifying dystonia and estimating its severity during routine clinical care. Clinical dystonia severity assessment could be guided by assessing specific dystonic movements, the consistency with which they are triggered, and their functional impact.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"174 ","pages":"Pages 126-134"},"PeriodicalIF":2.1,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145506326","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cannabidiol as Adjunctive Treatment in Drug-Resistant Epilepsy With Epileptic Spasms Beyond Two Years of Age","authors":"Elena González-Alguacil MD,&nbsp;J. Jose García Peñas MD,&nbsp;Nuria Lamagrande Casanova MD,&nbsp;Elsa Maria Santana Cabrera MD,&nbsp;Anna Duat Rodríguez PhD,&nbsp;Víctor Soto Insuga PhD","doi":"10.1016/j.pediatrneurol.2025.10.013","DOIUrl":"10.1016/j.pediatrneurol.2025.10.013","url":null,"abstract":"<div><h3>Background</h3><div>To assess the efficacy and safety of adjunctive cannabidiol (CBD) in children with drug-resistant epileptic spasms (ES) beyond age 2 years.</div></div><div><h3>Methods</h3><div>We conducted a retrospective longitudinal study of patients with childhood epileptic spasms (CESs) treated with purified CBD (Epidyolex) at Niño Jesus Hospital in Madrid, Spain, from 2020 to 2024. All patients were older than 2 years and had drug-resistant epilepsy and ES as the primary seizure type. Efficacy was assessed by comparing ES frequency before and after CBD treatment, with a good response defined as a ≥50% reduction in ES. Adverse effects (AEs) were recorded to assess safety and tolerability.</div></div><div><h3>Results</h3><div>Of the 53 patients included, 58.5% achieved a ≥50% reduction in ES frequency, 15% of whom attained complete freedom from ES. Patients with malformations of cortical development and Down syndrome showed the highest response rates. Clobazam was the most frequently associated medication (77.3%), indicating a potential synergistic effect. AEs were reported in 62.2% of patients, with drowsiness, hyperammonemia, and behavioral disturbances being the most common. CBD was discontinued in 17% due to lack of efficacy and in 11.3% due to AEs.</div></div><div><h3>Conclusions</h3><div>CBD appears to be an effective and well-tolerated adjunctive treatment for drug-resistant ES in children older than 2 years. Significant response rates were found, particularly in patients with Down syndrome and malformations of cortical development. Future studies with larger cohorts and longer follow-up are needed to validate these findings and explore the potential for earlier use of CBD in treatment-resistant epilepsy.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"174 ","pages":"Pages 81-85"},"PeriodicalIF":2.1,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145459190","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Rituximab Administration in Kleine–Levin Syndrome: A Case Report","authors":"Naoki Yamada MD,&nbsp;Ichiro Kuki MD, PhD,&nbsp;Masataka Fukuoka MD,&nbsp;Megumi Nukui MD,&nbsp;Takeshi Inoue MD,&nbsp;Shin Okazaki MD","doi":"10.1016/j.pediatrneurol.2025.10.008","DOIUrl":"10.1016/j.pediatrneurol.2025.10.008","url":null,"abstract":"","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"174 ","pages":"Pages 62-64"},"PeriodicalIF":2.1,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145438826","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Impacts of a Novel Pediatric Neuropalliative Medicine Clinic: Characterization of Referrer Feedback","authors":"Grant L. Lin MD, PhD ,&nbsp;Lauren Treat MD","doi":"10.1016/j.pediatrneurol.2025.10.018","DOIUrl":"10.1016/j.pediatrneurol.2025.10.018","url":null,"abstract":"<div><h3>Background</h3><div>Child neurologists provide care for children with complex chronic health conditions often accompanied by logistical and emotional complexities, as well as acute decision-making scenarios. Pediatric neuropalliative medicine is a nascent subspecialty that aims to augment standard child neurology practice by attending to not only the physical domains of neurologic disease but also the nonphysical issues that accompany advancing illness. The perceptions of clinicians who refer families for this kind of care have not previously been elucidated.</div></div><div><h3>Methods</h3><div>This survey project queried referrers to a newly established pediatric neuropalliative medicine clinic to characterize their perceptions, including barriers and facilitators of referral and the impact of this service line on clinicians. Specifically, the authors sought to understand whether neurologists viewed this clinic differently than other referring clinicians.</div></div><div><h3>Results</h3><div>A total of 94 surveys were completed, with 22% (n = 21) of respondents being neurology clinicians and 78% (n = 73) representing other pediatric specialties. Neurology clinicians were significantly more likely to report a preference for a neurologist in the palliative medicine role (76% of neurologists vs 16% of non-neurologists, <em>P</em> &lt; 0.001). While all referrers identified at least one benefit of referral to this clinic, neurologists were more likely to report that this new resource positively impacted their understanding of palliative medicine and their ability to devote more energy to their area of focus.</div></div><div><h3>Conclusions</h3><div>Pediatric neuropalliative expertise is valued by referring clinicians, with the greatest impact described by neurologists. Referrers noted benefit to patient care as well as enhancement in professional domains.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"174 ","pages":"Pages 140-146"},"PeriodicalIF":2.1,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145517803","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Verena Staedtke, MD, PhD (July 14, 1980 – March 31, 2025)","authors":"Carl E. Stafstrom MD, PhD","doi":"10.1016/j.pediatrneurol.2025.10.019","DOIUrl":"10.1016/j.pediatrneurol.2025.10.019","url":null,"abstract":"","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"174 ","pages":"Page 147"},"PeriodicalIF":2.1,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145517804","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}